Types of Ataxia - Ataxia UK

types of ataxia

Types of Ataxia

Ataxia is an umbrella term for a group of neurological disorders that affect balance, coordination and speech. Some types of ataxia such as Friedreich's ataxia and the spinocerebellar ataxias are inherited, meaning they are caused by genes passed on by parents to their children. When ataxia is not inherited there can be a number of different causes, identified (brain damage, diet, drug side effects) or not (idiopathic).

To find out more information on the ataxias, please take a look at our 'Ataxia: what's that?' booklet.

To find out information on specific types of ataxia, please click on the specific type below.

If you cannot find the information that you are looking for here, you can call our helpline on 0800 995 6037 or email helpline@ataxia.org.uk

Friedreich's ataxia is the most common form of hereditary ataxia, and is caused by an inherited mutation in the frataxin (FXN) gene. See our information booklet for more information:

Friedreich's ataxia

As well as becoming a Friend of Ataxia UK, we encourage anyone with a diagnosis of Friedreich's ataxia to join the FA Global Patient Registry.

The Spinocerebellar ataxias (also called SCA's) are caused by mutations in different genes. For example, spinocerebellar ataxia 1 is linked to a default in the gene SCA1.

Through research, more than 100 types of SCA's have been discovered since 1965. For more information on the different types of Spinocerebellar ataxias, see below:

Spinocerebellar ataxia 1 (SCA1)

Spinocerebellar ataxia 2 (SCA2)

Spinocerebellar ataxia 3 (SCA3)

Spinocerebellar ataxia 6 (SCA6)

Spinocerebellar ataxia 7 (SCA7)

Spinocerebellar ataxia 11 (SCA11)

Ataxia can be acquired during someone's lifetime, and caused by something other than a genetic malfunction.

Strokes, gluten intolereance, alcohol, vitamin-E deficiency and trauma to the head can all cause ataxia. Some of these types can be reversed, depending on the cause; a vitamin-E deficiency can be reduced by attention given to diet, for example, that will reduce ataxic symptoms and in some cases, eliminate them all together. A patient must consult their GP or neurologist before attempting to make any changes to their diet.

Idiopathic ataxia is the name we give a type of ataxia for which the cause is not yet known. Almost 50% of people with the condition have idiopathic ataxia, showing just how much more work there is to do.

Gluten ataxia is caused by a sensitivity to the protein gluten found in wheat products. It is one of the most common forms of sporadic idiopathic ataxia (where ataxia is found in people with no family history of ataxia and no known cause). You can find more information on Gluten ataxia research here.

Our specialist ataxia neurologist, Professor M Hadjivassiliou, is a leading expert in gluten ataxia. He runs the Specialist Ataxia Centre in Sheffield, and is leading research into the condition. If you have a diagnosis of gluten ataxia, please ask for a referral from your GP to the centre for specialist care. You can read how to do that here.

There is also a useful app that helps to monitor the gluten content in well-known products, assisting with your shopping and taking control over your condition.

Episodic ataxia is characterised as bouts or attacks of ataxia symptoms. The episodic ataxias are a relatively rare group of conditions which, as their name suggests, tend to affect people in bouts or attacks of unsteadiness. There are several types, but type 2 are the most well understood, and they are called episodic ataxia type 1 and 2. Both of these occur in families and are inherited in what is known as an autosomal dominant manner. This means that an affected individual has a 50% chance of passing the gene on to their children.

You can read more about the episodic ataxias here.

There are some other forms of ataxia, such as those below:

Ataxia with oculomotor apraxia (AOA) comes in two forms, AOA1 or AOA2. They make two of the rarest types of ataxia:


Dentatorubral pallidoluysian atrophy (DRPLA) is a type of inherited progressive late-onset cerebellar ataxia. It is characterised by a defect in a gene that causes damage to a certain part of the brain, the cerebellum.

CANVAS (Cerebellar ataxia, neuropathy and vestibular areflexia syndrome)

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